Blastic natural killer cell lymphoma/leukemia (CD56-positive blastic tumor): Prognostication and categorization according to anatomic sites of involvement

Ritsuro Suzuki, Shigeo Nakamura, Junji Suzumiya, Koichi Ichimura, Masako Ichikawa, Kiyoyuki Ogata, Yoshimasa Kura, Keiko Aikawa, Hirofumi Teshima, Masahiro Sako, Hiroshi Kojima, Mitsufumi Nishio, Tadashi Yoshino, Hiroki Sugimori, Keisei Kawa, Kazuo Oshimi

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Abstract

BACKGROUND. Elastic natural killer (NK) cell lymphoma/leukemia (BNKL) is an immature CD56-positive neoplasm, which was recognized recently and characterized by systemic proliferation of tumor cells including skin, lymph node, and bone marrow. METHODS. The current study analyzed 47 patients with BNKL (27 had leukemias and 20 had lymphomas). Patient data were collected for the survey of the NK-Cell Tumor Study Group. RESULTS. There were 33 males and 14 females, with a median age of 53 years (range, 3 months to 89 years). There were few clinicopathologic differences between the leukemia and lymphoma types. Cutaneous involvement was noted at diagnosis in 28 patients, who presented a tendency for older age of onset (median: 56 vs. 46 years, P = 0.11) than patients with noncutaneous BNKL. Cutaneous BNKL showed less frequent mediastinal involvement (4% vs. 53%, P = 0.0002) and less severe thrombocytopenia (P = 0.03). Phenotypic characteristics were also different, with cutaneous BNKL favoring CD4 and HLA-DR expression, and noncutaneous BNKL favoring CD16 and CD34 expression. Both groups responded well to chemotherapy for lymphoid malignancies, but disease recurrence was frequent. The prognosis of patients with noncutaneous BNKL was significantly poorer than that of patients with cutaneous BNKL (median survival: 15 vs. 25 months, P = 0.02). Multivariate analysis confirmed that cutaneous involvement was a significant and independent prognostic factor for BNKL, as were age of onset and leukocyte count. CONCLUSIONS. These findings suggested that BNKL is a heterogeneous disease and contains at least two subtypes. Although further investigations are needed to settle a marker for distinction, the presence of cutaneous involvement is a useful prognostic factor.

Original languageEnglish
Pages (from-to)1022-1031
Number of pages10
JournalCancer
Volume104
Issue number5
DOIs
Publication statusPublished - Sep 1 2005

Keywords

  • CD56
  • Lymphoblastic
  • Lymphoma
  • Natural killer cell
  • Terminal deoxynucleotidyl transferase

ASJC Scopus subject areas

  • Oncology
  • Cancer Research

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    Suzuki, R., Nakamura, S., Suzumiya, J., Ichimura, K., Ichikawa, M., Ogata, K., Kura, Y., Aikawa, K., Teshima, H., Sako, M., Kojima, H., Nishio, M., Yoshino, T., Sugimori, H., Kawa, K., & Oshimi, K. (2005). Blastic natural killer cell lymphoma/leukemia (CD56-positive blastic tumor): Prognostication and categorization according to anatomic sites of involvement. Cancer, 104(5), 1022-1031. https://doi.org/10.1002/cncr.21268